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Pemphigus vulgaris: an acquired blistering disease
Kirby J Scott1, Brian J McKinnon
1Department of Otolaryngology-Head and Neck Surgery, Naval Medical Center, Portsmouth, VA 23708-5000, USA. KJScott@mar.med.navy.mil
Abstract:
Pemphigus vulgaris is one of a group of autoimmune disorders that are caused by autoantibodies against the desmoglein adhesion molecules of squamous epithelial cells. It is a rare form of immune dysfunction that can prove vexing to the patient and physician, but it has distinct clinical and histologic findings. We report a case of a patient with the autoimmune blistering disease pemphigus vulgaris localized to the oral cavity and discuss the important clinical, immunopathologic, and therapeutic factors of this disease. This case report highlights the unusual nature of pemphigus vulgaris, the modalities used in its diagnosis, and effectiveness of therapy. Pemphigus vulgaris is an uncommon disease blistering disorder due to desmoglein autoantibodies, whose presentation can be alarming and puzzling to the clinician. Awareness of the disease's presentation and mechanism will allow for an efficient diagnostic evaluation and timely treatment.